**5. Clinical presentation and physical exam**

A thorough history and physical exam is necessary to accurately diagnose Dupuytren's disease, which has a classic presentation. It is important to assess risk factors including family history, northern European descent, smoking, alcohol use, history of diabetes mellitus, or previous trauma to the hand in order to gain a broader picture of the patients' presentation. In many cases, disease is bilateral with one hand affected more than the other so it important to evaluate both hand. The ring finger is the most commonly affected digit followed by the small, middle, index, and thumb [48]. Other fibroproliferative diseases have been associated with Dupuytren's disease and include Garrod's nodes, Ledderhose disease, and Peyronie's disease. Garrod's nodes, also called knuckle pads, can be visualized on the dorsal PIP joints and are subcutaneous nodules that histologically portray myofibroblasts proliferation. Ledderhose is a fibrosis of the plantar fascia and Peyronie's disease is an inflammation and scarring of the tunica albuginea of the penis. Rayan et al. described three phases to Dupuytren's disease clinical presentation: the early, intermediate, and late phases [49]. These three phases provide a good framework to assess the patient's disease status, and each phase is characterized by distinct aspects of the disease.

#### **5.1. Early phase**

Early disease is characterized by skin dimpling, puckering, and pitting, usually on the medial aspect of the palm. These changes can lead to the patient seeking medical attention, however are also easily ignored by some patients. A Dupuytren's patient population study suggests that approximately 11% of Dupuytren's disease patients seek attention from their physician with a chief complaint of skin changes on the palm [12]. Physical exam during the early phase of disease can confirm these skin changes upon inspection. Pitting of skin on the medial palm of the hand is a good indication of developing Dupuytren's disease. Palpation reveals thickening and dimpling of the skin around the effected joint. The underlying fat on the medial palm becomes fibrotic near the distal palmar crease. Active range of motion (ROM) and strength testing in early disease will reveal no limitations, though more severe skin adhesions can lead to a slight decrease in mobility and function of the affected digits in some patients.

#### **5.2. Intermediate phase**

to the development of Dupuytren's disease. Research has also demonstrated prostaglandins PGE2 and PGF2α play a role in contractility of smooth muscle associated with myofibroblasts [44, 47]. This contractile influence on myofibroblasts is thought to contribute to the contraction of tissue late in the disease. The source of these prostaglandins are possibly from microcircula-

Studies have suggested an immune mediated response in the pathophysiology of Dupuytren's disease. Mayerl et al. describe abundant accumulation of immune cells in Dupuytren's tissue, including mononuclear CD3+, CD4+ > CD8+, and primarily a Th1 mediated response [39]. These clusters of immune cells were found around blood vessels in the area, suggesting the fibroproliferation exists in Dupuytren's may be due to microvascular damage mediated by the immune system. Further research is required to determine the relationship of Dupuytren's to

A thorough history and physical exam is necessary to accurately diagnose Dupuytren's disease, which has a classic presentation. It is important to assess risk factors including family history, northern European descent, smoking, alcohol use, history of diabetes mellitus, or previous trauma to the hand in order to gain a broader picture of the patients' presentation. In many cases, disease is bilateral with one hand affected more than the other so it important to evaluate both hand. The ring finger is the most commonly affected digit followed by the small, middle, index, and thumb [48]. Other fibroproliferative diseases have been associated with Dupuytren's disease and include Garrod's nodes, Ledderhose disease, and Peyronie's disease. Garrod's nodes, also called knuckle pads, can be visualized on the dorsal PIP joints and are subcutaneous nodules that histologically portray myofibroblasts proliferation. Ledderhose is a fibrosis of the plantar fascia and Peyronie's disease is an inflammation and scarring of the tunica albuginea of the penis. Rayan et al. described three phases to Dupuytren's disease clinical presentation: the early, intermediate, and late phases [49]. These three phases provide a good framework to assess the patient's disease status, and each phase is characterized by

Early disease is characterized by skin dimpling, puckering, and pitting, usually on the medial aspect of the palm. These changes can lead to the patient seeking medical attention, however are also easily ignored by some patients. A Dupuytren's patient population study suggests that approximately 11% of Dupuytren's disease patients seek attention from their physician with a chief complaint of skin changes on the palm [12]. Physical exam during the early phase of disease can confirm these skin changes upon inspection. Pitting of skin on the medial palm of the hand is a good indication of developing Dupuytren's disease. Palpation reveals thickening and

tion and perinodular fat, as nodules are highly vascularized and fatty.

*4.2.4. Immune mediated*

64 Essentials of Hand Surgery

an immune mediated response.

distinct aspects of the disease.

**5.1. Early phase**

**5. Clinical presentation and physical exam**

The appearance of Dupuytren's nodules and cords signifies the intermediate phase of disease. Nodule formation is often one of the first patient complaints and occurs during intermediate stages of the disease. Approximately 42% of patients with Dupuytren's disease present to the office due to a nodule [12]. Nodules most commonly form proximal to the palmar crease overlying the metacarpophalangeal joint of the affected digit, and encompass the superficial layers of the palmar and digital fascia. Sometimes digital nodules are seen at the base of the proximal interphalangeal joint. Though often painless, larger nodules can cause pain when they exert pressure on underlying flexor tendons. Painful, chronic nodules are more indicative of intrinsic joint disease and rheumatoid arthritis, and must be differentiated from a Dupuytren's disease nodule. After the appearance of a nodule, a pathologic cord may form within the palmar fascia. Approximately 12% of patients seek will seek care following development of a cord [12]. Nodules often regresses, but in some cases can be present simultaneously with Dupuytren's cords. Initial cords are often unnoticeable and blend in with the underlying connective tissue, but over time, they become thick and resemble subcutaneous tendon-like structures upon inspection (**Figure 1**). Palpation reveals an immobile, thickened cord. Cord formation is extremely variable in terms of location. The most common cords arise in the palm, and include peritendinous, natatory, and vertical cords arising from their respective bands in the palmar

**Figure 1.** A patient with Dupuytren's cords leading to contractures and affecting the bilateral small and ring fingers.

fascia. Digital cords frequently seen include central and spiral cords. Active and passive ROM testing during the intermediate disease will often reveal no limitations in patients with nodules, but as cords form patients will begin to lose extension of the involved joint.

The degree of extension deficit is taken into account when staging Dupuytren's disease. The adapted Tubiana staging system is the most common method of classifying the progression

Dupuytren's Disease

67

http://dx.doi.org/10.5772/intechopen.72759

Dupuytren's disease is a clinical diagnoses based on a patient's history and physical exam. Its hallmark features consist of an indolent, progressive course characterized by palmar skin changes, painless nodules, and fibrotic cords leading to flexion contractures of the digits. Patients presenting with later findings of the disease consisting of fibrotic cords and contracted digits are more clearly diagnosed. Conversely, patients presenting with earlier features of Dupuytren's disease such as painless nodules may not be as easily distinguished from other diseases. Stenosing tenosynovitis, also known as a trigger finger, and soft tissue tumors may be mistaken for Dupuytren's disease. Stenosing tenosynovitis can be differentiated from Dupuytren's by tenderness over the A1 pulley with symptomatic locking or triggering of the digit and often no ROM deficit. Soft tissue masses typically do not present with skin thickening and pitting as seen in Dupuytren's disease. In addition, Dupuytren's nodules are often fixed to the skin and palmar fascia. Early Dupuytren's disease may be difficult to distinguish from diabetic cheiroarthropathy, however, involvement of multiple digits and a waxy appearance of the skin are clues to distinguish diabetic cheiroarthropathy. Other pathologies that may present with some features similar to Dupuytren's disease include: ulnar claw, rheumatoid arthritis, Volkmann's contracture, and camptodactyly. Radiographs should be considered in patients presenting with a history of trauma to rule out a fracture or dislocation. Further diagnostic imaging such as MRI may be considered in special cases to rule out suspicion of other disease processes, but is not required to diagnose Dupuytren's disease. A thorough history and physical exam is key to accurately diagnosing Dupuytren's disease.

Despite the recent advances in understanding the pathophysiology of Dupuytren's disease the treatment options remain palliative and not curative. Non-operative treatment is recommended in patients with isolated disease without contractures and in patients with mild contractures without significant interference with activities of daily living. Observation is a reasonable non-operative option for many patients with early disease and minimal symptoms. Studies have estimated about 50.8% of patients with palpable nodules will progress to developing cords after 8 years from diagnosis, and of these only 17% will develop contrac-

Surgery is the mainstay treatment for Dupuytren's contractures. However, non-operative interventions continue to be pursued as an alternative option to surgical intervention. Splinting and physical therapy have mostly been utilized as a post-operative intervention to prevent recurrence. Critics of splinting and physical therapy often express concern it may worsen the contracture if the contractile tissue is not first removed. In vitro studies have reported

of Dupuytren's disease (**Table 1**) [51, 52].

**7. Nonoperative treatment**

tures meeting criteria for surgical intervention [53].

**6. Diagnosis**

#### **5.3. Late phase**

Late disease is defined by contraction of cords and the classic "bent finger" appearance of Dupuytren's disease (**Figure 2**). Approximately 10% of Dupuytren's disease patients will present during the late stage of the disease complaining of a permanent bent finger [12]. Contracture of the MCP joint often occurs before the PCP joint. Contractures often lead to difficulties in activates of daily living and patients will report difficulties with chores, washing, putting a hand in a pocket, and handshakes. Inspection and palpation will reveal a contracted, fibrotic cord. Both active and passive finger extension of the effected finger will likely be impaired, the extent of which is determined by severity of disease. Pain with ROM is rarely reported and if present should prompt further evaluation. The table top test was described in 1982 by Hueston and is specific to a Dupuytren's diagnosis and has been used to stage disease progression [50]. The test involves placing the patient's hand on a tabletop with the palmar side down. The test is positive if the patient cannot flatten the hand against the table and is indicative of the late phase of the disease.

**Figure 2.** A patient with Dupuytren's PIP joint contracture with a flexion contracture of approximately 100°.


**Table 1.** Tubiana staging system based on a digit's total extension deficit [51, 52].

The degree of extension deficit is taken into account when staging Dupuytren's disease. The adapted Tubiana staging system is the most common method of classifying the progression of Dupuytren's disease (**Table 1**) [51, 52].
